(S)-beta-Aminoisobutyric acid (BioDeep_00000014452)
Secondary id: BioDeep_00000875074, BioDeep_00000897250, BioDeep_00001868745, BioDeep_00001890956
human metabolite PANOMIX_OTCML-2023 Endogenous blood metabolite Volatile Flavor Compounds
代谢物信息卡片
化学式: C4H9NO2 (103.0633254)
中文名称: L-3-氨基异丁酸, S-3-氨基异丁酸
谱图信息:
最多检出来源 Homo sapiens(blood) 12%
分子结构信息
SMILES: CC(CN)C(=O)O
InChI: InChI=1S/C4H9NO2/c1-3(2-5)4(6)7/h3H,2,5H2,1H3,(H,6,7)/t3-/m0/s1
描述信息
beta-Aminoisobutyric acid is a non-protein amino acid originating from the catabolism of thymine and valine. The concentration of beta-aminoisobutyric acid is normally low in urine as beta-aminoisobutyric acid is further catabolized by beta-aminoisobutyrate aminotransferases to methylmalonic acid semialdehyde and propionyl-CoA. beta-Aminoisobutyric acid occurs in two isomeric forms and both enantiomers of beta-aminoisobutyric acid can be detected in human urine and plasma. In plasma, the S-enantiomer is the predominant type due to active renal reabsorption. In contrast, urine almost exclusively contains the R-enantiomer of beta-aminoisobutyric acid, which is eliminated both by filtration and tubular secretion. Persistently increased levels of beta-aminoisobutyric acid have been observed in individuals with a deficiency of R (-)-beta-aminoisobutyrate-pyruvate aminotransferase. In addition, transient high levels of beta-aminoisobutyric acid have been observed under a variety of pathological conditions such as lead poisoning, starvation, in total body irradiation, and in a number of malignancies. The S-enantiomer of beta-aminoisobutyric acid is predominantly derived from the catabolism of valine. It has been suggested that altered homeostasis of beta-alanine underlies some of the clinical abnormalities encountered in patients with a dihydropyrimidine dehydrogenase (DPD) deficiency. DPD constitutes the first step of the pyrimidine degradation pathway, in which the pyrimidine bases uracil and thymine are catabolized to beta-alanine and the R-enantiomer of beta-aminoisobutyric acid respectively. In normal individuals with an intact pyrimidine degradation pathway, R-methylmalonic acid semialdehyde can be synthesized directly from the catabolism of thymine. Hence, there might be less cross-over between the valine and thymine pathway, allowing the conversion of S-methylmalonic acid semialdehyde into S-beta-aminoisobutyric acid and the subsequent accumulation of S-beta-aminoisobutyric acid in plasma (PMID: 14705962, 14292857, 14453202).
(S)-b-aminoisobutyric acid is a non-protein amino acid originating from the catabolism of thymine and valine.
同义名列表
45 个代谢物同义名
(S)-3-amino-2-Methyl-propanoic acid; (2S)-3-amino-2-methylpropanoic acid; (S)-3-Amino-2-methylpropanoic acid; L-3-amino-2-Methylpropanoic acid; L-3-amino-2-Methylpropionic acid; 2S-methyl-3-amino-propanoic acid; (S)-3-amino-2-Methyl-propanoate; (S)-3-Amino-2-methylpropanoate; (+)-beta-Aminoisobutyric acid; (S)-beta-Aminoisobutyric acid; (+)-alpha-Methyl-beta-alanine; (S)-3-Amino-isobutanoic acid; L-3-amino-2-Methylpropanoate; (S)-b-amino-isobutyric acid; S-beta-Aminoisobutyric acid; (S)-3-Aminoisobutanoic acid; L-beta-Aminoisobutyric acid; (S)-3-Amino-isobutyric acid; (+)-b-Aminoisobutyric acid; (S)-3-Aminoisobutyric acid; L-3-Amino-isobutanoic acid; (S)-Β-aminoisobutyric acid; (S)-b-aminoisobutyric acid; L-3-Amino-isobutyric acid; (S)-beta-Aminoisobutyrate; L-b-Aminoisobutyric acid; L-β-aminoisobutyric acid; L-3-Aminoisobutyric acid; (S)-3-Amino-isobutanoate; L-beta-Aminoisobutyrate; S-beta-Aminoisobutyrate; (S)-3-Aminoisobutanoate; (S)-3-Amino-isobutyrate; L-2-Methyl-beta-alanine; L-3-Amino-isobutanoate; (S)-3-Aminoisobutyrate; (+)-a-Methyl-b-alanine; (S)-b-Aminoisobutyrate; (S)-Β-aminoisobutyrate; L-3-Amino-isobutyrate; L-3-Aminoisobutyrate; S-b-Aminoisobutyrate; L-2-Methyl-b-alanine; L-b-Aminoisobutyrate; L-3-Aminoisobutyric acid
数据库引用编号
19 个数据库交叉引用编号
- ChEBI: CHEBI:33094
- KEGG: C03284
- PubChem: 439434
- HMDB: HMDB0002166
- Metlin: METLIN45884
- MetaCyc: CPD-466
- foodb: FDB022878
- chemspider: 388543
- CAS: 4249-19-8
- ChEBI: CHEBI:18188
- PubChem: 6143
- LipidMAPS: LMFA01100050
- PDB-CCD: 62H
- PDB-CCD: BIB
- 3DMET: B01645
- NIKKAJI: J52.799H
- medchemexpress: HY-113380
- LOTUS: LTS0136817
- wikidata: Q27088673
分类词条
相关代谢途径
Reactome(0)
BioCyc(0)
PlantCyc(0)
代谢反应
53 个相关的代谢反应过程信息。
Reactome(0)
BioCyc(0)
WikiPathways(0)
Plant Reactome(0)
INOH(0)
PlantCyc(0)
COVID-19 Disease Map(0)
PathBank(53)
- Valine, Leucine, and Isoleucine Degradation:
-Ketoisovaleric acid + Thiamine pyrophosphate ⟶ 2-Methyl-1-hydroxypropyl-ThPP + Carbon dioxide
- beta-Ketothiolase Deficiency:
-Ketoisovaleric acid + Thiamine pyrophosphate ⟶ 2-Methyl-1-hydroxypropyl-ThPP + Carbon dioxide
- 2-Methyl-3-hydroxybutyryl-CoA Dehydrogenase Deficiency:
-Ketoisovaleric acid + Thiamine pyrophosphate ⟶ 2-Methyl-1-hydroxypropyl-ThPP + Carbon dioxide
- Propionic Acidemia:
-Ketoisovaleric acid + Thiamine pyrophosphate ⟶ 2-Methyl-1-hydroxypropyl-ThPP + Carbon dioxide
- 3-Hydroxy-3-methylglutaryl-CoA Lyase Deficiency:
-Ketoisovaleric acid + Thiamine pyrophosphate ⟶ 2-Methyl-1-hydroxypropyl-ThPP + Carbon dioxide
- Maple Syrup Urine Disease:
-Ketoisovaleric acid + Thiamine pyrophosphate ⟶ 2-Methyl-1-hydroxypropyl-ThPP + Carbon dioxide
- 3-Methylcrotonyl-CoA Carboxylase Deficiency Type I:
-Ketoisovaleric acid + Thiamine pyrophosphate ⟶ 2-Methyl-1-hydroxypropyl-ThPP + Carbon dioxide
- 3-Methylglutaconic Aciduria Type I:
-Ketoisovaleric acid + Thiamine pyrophosphate ⟶ 2-Methyl-1-hydroxypropyl-ThPP + Carbon dioxide
- 3-Methylglutaconic Aciduria Type III:
-Ketoisovaleric acid + Thiamine pyrophosphate ⟶ 2-Methyl-1-hydroxypropyl-ThPP + Carbon dioxide
- Methylmalonate Semialdehyde Dehydrogenase Deficiency:
-Ketoisovaleric acid + Thiamine pyrophosphate ⟶ 2-Methyl-1-hydroxypropyl-ThPP + Carbon dioxide
- Methylmalonic Aciduria:
-Ketoisovaleric acid + Thiamine pyrophosphate ⟶ 2-Methyl-1-hydroxypropyl-ThPP + Carbon dioxide
- Isovaleric Aciduria:
-Ketoisovaleric acid + Thiamine pyrophosphate ⟶ 2-Methyl-1-hydroxypropyl-ThPP + Carbon dioxide
- 3-Methylglutaconic Aciduria Type IV:
-Ketoisovaleric acid + Thiamine pyrophosphate ⟶ 2-Methyl-1-hydroxypropyl-ThPP + Carbon dioxide
- 3-Hydroxyisobutyric Acid Dehydrogenase Deficiency:
-Ketoisovaleric acid + Thiamine pyrophosphate ⟶ 2-Methyl-1-hydroxypropyl-ThPP + Carbon dioxide
- 3-Hydroxyisobutyric Aciduria:
-Ketoisovaleric acid + Thiamine pyrophosphate ⟶ 2-Methyl-1-hydroxypropyl-ThPP + Carbon dioxide
- Isobutyryl-CoA Dehydrogenase Deficiency:
-Ketoisovaleric acid + Thiamine pyrophosphate ⟶ 2-Methyl-1-hydroxypropyl-ThPP + Carbon dioxide
- Isovaleric Acidemia:
-Ketoisovaleric acid + Thiamine pyrophosphate ⟶ 2-Methyl-1-hydroxypropyl-ThPP + Carbon dioxide
- Valine Degradation:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
- Valine, Leucine, and Isoleucine Degradation:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
- 2-Methyl-3-hydroxybutryl-CoA Dehydrogenase Deficiency:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
- 3-Hydroxy-3-methylglutaryl-CoA Lyase Deficiency:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
- 3-Methylcrotonyl-CoA Carboxylase Deficiency Type I:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
- 3-Methylglutaconic Aciduria Type I:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
- 3-Methylglutaconic Aciduria Type III:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
- 3-Methylglutaconic Aciduria Type IV:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
- beta-Ketothiolase Deficiency:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
- Isovaleric Aciduria:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
- Maple Syrup Urine Disease:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
- Methylmalonate Semialdehyde Dehydrogenase Deficiency:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
- Methylmalonic Aciduria:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
- Propionic Acidemia:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
- 3-Hydroxyisobutyric Acid Dehydrogenase Deficiency:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
- 3-Hydroxyisobutyric Aciduria:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
- Isobutyryl-CoA Dehydrogenase Deficiency:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
- Isovaleric Acidemia:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
- Valine, Leucine, and Isoleucine Degradation:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
- Valine, Leucine, and Isoleucine Degradation:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
- 2-Methyl-3-hydroxybutryl-CoA Dehydrogenase Deficiency:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
- 3-Hydroxy-3-methylglutaryl-CoA Lyase Deficiency:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
- 3-Methylcrotonyl-CoA Carboxylase Deficiency Type I:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
- 3-Methylglutaconic Aciduria Type I:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
- 3-Methylglutaconic Aciduria Type III:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
- 3-Methylglutaconic Aciduria Type IV:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
- beta-Ketothiolase Deficiency:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
- Isovaleric Aciduria:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
- Maple Syrup Urine Disease:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
- Methylmalonate Semialdehyde Dehydrogenase Deficiency:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
- Methylmalonic Aciduria:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
- Propionic Acidemia:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
- 3-Hydroxyisobutyric Acid Dehydrogenase Deficiency:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
- 3-Hydroxyisobutyric Aciduria:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
- Isobutyryl-CoA Dehydrogenase Deficiency:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
- Isovaleric Acidemia:
L-Valine + Oxoglutaric acid ⟶ -Ketoisovaleric acid + L-Glutamic acid
PharmGKB(0)
1 个相关的物种来源信息
在这里通过桑基图来展示出与当前的这个代谢物在我们的BioDeep知识库中具有相关联信息的其他代谢物。在这里进行关联的信息来源主要有:
- PubMed: 来源于PubMed文献库中的文献信息,我们通过自然语言数据挖掘得到的在同一篇文献中被同时提及的相关代谢物列表,这个列表按照代谢物同时出现的文献数量降序排序,取前10个代谢物作为相关研究中关联性很高的代谢物集合展示在桑基图中。
- NCBI Taxonomy: 通过文献数据挖掘,得到的代谢物物种来源信息关联。这个关联信息同样按照出现的次数降序排序,取前10个代谢物作为高关联度的代谢物集合展示在桑吉图上。
- Chemical Taxonomy: 在物质分类上处于同一个分类集合中的其他代谢物
- Chemical Reaction: 在化学反应过程中,存在为当前代谢物相关联的生化反应过程中的反应底物或者反应产物的关联代谢物信息。
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